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17 Possible Causes for 3-4 Hz Spikes, Mild Cerebellar Hypoplasia

  • Benign Adult Familial Myoclonic Epilepsy

    hypoplasia-hydranencephaly syndrome Multiple acyl-CoA dehydrogenase deficiency Multiple acyl-CoA dehydrogenase deficiency, mild type Multiple acyl-CoA dehydrogenase deficiency[orpha.net] Childhood absence epilepsy with tonic-clonic seizures and electroencephalogram 3-4-Hz spike and multispike-slow wave complexes: linkage to chromosome 8q24.[reference.medscape.com] Her EEG showed 34 Hz spike and wave discharges. She had a daughter with childhood absence epilepsy, and two sisters and a cousin with seizures (fig 2 , family 1).[jnnp.bmj.com]

  • West Syndrome

    Magnetic resonance imaging of the brain revealed mild cerebral and cerebellar atrophy, high-signal-intensity areas in the white matter, and hypoplasia of the corpus callosum[ncbi.nlm.nih.gov]

  • Unverricht-Lundborg Syndrome

    hypoplasia-hydranencephaly syndrome Multiple acyl-CoA dehydrogenase deficiency Multiple acyl-CoA dehydrogenase deficiency, mild type Multiple acyl-CoA dehydrogenase deficiency[orpha.net] .  EEG - diffuse slow background activity and generalised high-voltage spike and wave, and polyspike and wave paroxysms, ranging from 2–3 Hz to 4–6 Hz, which reach a maximum[slideshare.net] Multiloculated renal cyst Multiminicore disease Multiminicore myopathy Multinodular goiter-cystic kidney-polydactyly syndrome Multinucleated neurons-anhydramnios-renal dysplasia-cerebellar[orpha.net]

  • Lissencephaly

    hypoplasia TUBA1A AD TUBB2B Lissencephaly with cerebellar hypoplasia, reelinopathy type Mild frontal lissencephaly w/severe cerebellar hypoplasia RELN AR VLDLR Cobblestone[ncbi.nlm.nih.gov] Childhood absence epilepsy with tonicclonic seizures and electroencephalogram 3-4-Hz spike and multispikeslow wave complexes: linkage to chromosome 8q24.[books.google.es] Cerebellar hypoplasia is often associated with type 2 lissencephaly; however, our cases showed no polymicrogyria, and their clinical findings were quite mild compared with[candicelange.com]

  • Neuronal Ceroid Lipofuscinosis

    cerebellar ataxia and white matter edema Leukonychia totalis Leukotriene C4 synthase deficiency Levine-Critchley syndrome Levy-Hollister syndrome Lewandowsky-Lutz syndrome[csbg.cnb.csic.es] Photic stimulation at 1 to 3 Hz elicited discrete spike and wave discharges in the electroencephalogram, which were diminished at a higher frequency of stimulation.[ncbi.nlm.nih.gov] Leydig cel hypoplasia due to complete LH receptor inactivation Leydig cel hypoplasia due to complete luteinizing hormone receptor inactivation Leydig cel hypoplasia due to[csbg.cnb.csic.es]

  • Angelman Syndrome

    Cerebellar hypoplasia Seizures Pathophysiology [ edit ] Adenylosuccinate lyase deficiency is responsible for a range of symptoms that involve psychomotor retardation, often[en.wikipedia.org] High amplitude rhythmic 4-6 Hz activity, prominent in the occipital regions, with spikes, which can be facilitated by eye closure, is often seen in children under the age[ncbi.nlm.nih.gov] […] to 6 Hz theta with centrotemporal emphasis; or c) high amplitude 3 to 6 Hz rhythmic activity containing small spike over posterior regions What types of care and treatment[epilepsy.com]

  • Adult-Onset Autosomal Recessive Cerebellar Ataxia

    […] ataxia and mental retardation known as the disequilibrium syndrome. 36, 45 Imaging shows significant cerebellar hypoplasia and mild cerebral cortex thickening and gyral simplification[ajnr.org] There is a burst of generalized discharges of spikes, multiple spikes and slow waves at 4 to 5 Hz, with greater voltage on the left.[academic.oup.com] Other inherited diseases that cause nonprogressive ataxia with mild-to-moderate vermis hypoplasia include OPHN1 and Opitz/GBBB syndromes. 6 Although the phenotype is variable[ajnr.org]

  • Autosomal Recessive Spinocerebellar Ataxia 8

    cerebellar hypoplasia or vermian aplasia • Joubert syndrome – absence of cerebellar vermis and “molar tooth sign” at cerebellar & mid brain junction • Fragile X associated[slideshare.net] There is a burst of generalized discharges of spikes, multiple spikes and slow waves at 4 to 5 Hz, with greater voltage on the left.[academic.oup.com] Neuro-radiology • FRDA- cervical spinal cord atrophy, mild cerebellar atrophy • Predominant vermian atrophy – AR ataxia- AT, AOA1, AOA2, Cayman Ataxia, SCAN • Congenital ataxia[slideshare.net]

  • Warburg Micro Syndrome 3

    MRI showed bilateral frontoparietal polymicrogyria extending to the insula and temporal gyri, cortical atrophy, delayed myelinization, hypoplasia of the cerebellar vermis[bio2rdf.org] Interictal EEG showed an intensification of the frontal-central-parietal focus of 3-4 Hz high amplitude activity, at times intermingled with synchronous or asynchronous spike-waves[omicsonline.org] […] and corpus callosum, and mild ventriculomegaly. mapping [omim_vocabulary:mapping] {2:Bem et al. (2011)} performed a genomewide linkage scan in 5 large consanguineous families[bio2rdf.org]

  • Miller Dieker Syndrome

    hypoplasia TUBA1A AD TUBB2B Lissencephaly with cerebellar hypoplasia, reelinopathy type Mild frontal lissencephaly w/severe cerebellar hypoplasia RELN AR VLDLR Cobblestone[ncbi.nlm.nih.gov] High amplitude 2-3 Hz frontal predominant activity may be seen; symmetrical 4-6 Hz high voltage activity or 3-6 Hz occipital activity overlaid with spikes and sharp waves[epilepsydiagnosis.org] (the anterior portion often appears flattened) Cavum septi pellucidi et vergae Normal brain stem and cerebellum in most individuals, mild cerebellar vermis hypoplasia in[ncbi.nlm.nih.gov]

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